Khaled Sadek
Mind Map by Khaled Sadek, updated more than 1 year ago
Khaled Sadek
Created by Khaled Sadek over 3 years ago


Mind Map on SCID, created by Khaled Sadek on 12/11/2016.

Resource summary

1 • Types of primary immunodeficiencies
1.1 Antibody
1.2 Cellular, combined
1.3 Phagocyte
1.4 Complement
1.5 Malfunction of regulation
1.6 Syndromes with compromised DNA repair
2 Inheritance of immunodeficiency
2.1 X-linked recessive
2.1.1 common in males more than females
3 What is SCID
3.1 Severe Combined Immunodeficiency
3.1.1 highly susceptible to life-threatening infections
3.2 a rare condition
3.3 Caused by defects in any of several possible genes
3.3.1 Adenosine deaminase deficiency ADA converts adenosine and deoxyadenosine to inosine and deoxyinosine Accumulation of dATP inhibition of ribonucleotide reductase B and T cells unable to divide
3.4 fatal, usually within the first year of life
3.5 Cellular and Humoral Components
3.5.1 Cellular Thymus not present T cells will not mature Thymus present but T cells are not produced Thymus will shrink
3.5.2 Humoral B cells affected from The absence of T cells cannot produce antibodies or cannot class switch
4 diagnosis of SCID
4.1 CBC + DBC
4.1.1 Leukopenia if below 3000 in children younger than 1 year
4.2 Total serum immunoglobulin (Ig) levels
4.2.1 IGM
4.3 Lymphocyte function
4.3.1 Mitogenic signals
4.4 Chest x-ray
4.4.1 thymus shadow
5 treatment and management of SCID
5.1 Protective management
5.1.1 Isolated bubble
5.2 IV Ig
5.3 Prophylaxis
5.4 Stopping live vaccinations
5.5 Hematopoietic cell transplant
5.5.1 First choice of treatment
5.6 Gene therapy
5.6.1 Not available for everyone
5.6.2 Very expensive
5.6.3 Limited studies done on it reducing its reliability
5.7 Enzyme replacement therapy ERT
5.7.1 Weekly IM
5.7.2 Might require continuous Ig therapy
6 Role of MHC (MAJOR Histocompatibility Complex) , HLA (Human Leukocyte Antigen)
6.1 Histocompatibility
6.1.1 Major Complex (MHC) Cluster of genes >>> Glycoproteins Discriminate self/ non-self Ag presenting structures Act as antigens in in-compatible recipient
6.1.2 Minor Antigen (miHA) Cell-surface receptors of donated organs Polymorphic proteins e.g. H-Y/ HA-2 A.A differences between donor & recipient
6.2 Classes of MHC Genes
6.2.1 Class I
6.2.2 Class II
6.2.3 Class III
6.3 Features of MHC
6.3.1 Highly Polymorphic
6.3.2 Co-dominant Expression
6.3.3 Low affinity, broad specificity
6.3.4 Low affinity, broad specificity
7 Tests for Compatibility
7.1 Blood Typing
7.2 Tissue Typing
7.2.1 Genomic typing: Allele specific PCR/Sequencing
7.3 Cross-Matching
7.3.1 Complement-dependent Cytotoxicity
7.3.2 Flow Cytometry
7.3.3 Mixed Lymphocyte Culture
7.3.4 Panel Reactive Antibody
7.4 Others
7.4.1 ELISA
7.4.2 Limiting Dilution Analysis
7.4.3 Direct Cytotoxicity Assay
8 Donor guidelines
8.1 Medical suitability
8.2 Donor eligibility
8.3 Donor evaluation
8.4 pathogens that may be transmitted by the graft
8.5 Informed consent
8.6 Preoperative assessment
9.1 Allograft
9.2 Definition
9.3 types
9.3.1 acute GVHD
9.3.2 Chronic GVHD
10 Post-transplant care recommendations
10.1 Long term screening
10.1.1 Weekly visits for one month
10.1.2 Biweekly visits in the next wo months
10.1.3 Monthly visit for six two twelve months
10.1.4 Vital signs, body weight and height in every visit
10.2 Vaccination
10.2.1 vaccines should be offered from about 12 months post HSCT
10.2.2 No live vaccines are given until at least 2 years post HCT
10.2.3 Same as defaulters schedule
10.2.4 patient’s surrounding family must be completely immunised
10.3 Screening for chronic GVHD
10.3.1 allogeneic hematopoietic stem cell transplant should be monitored for chronic GVHD
11 Consent in organ donation
11.1 Informed consent
11.2 Required request
11.3 Presumed consent
12 Organ donation in Islam
12.1 living donor
12.1.1 it is allowed to donate unless donor’s life is put at risk vital organs like the heart are not allowed to be donated physically cannot tolerate the operation
12.2 Dead donor
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